颅内非典型畸胎样横纹肌样瘤一例并文献复习

Clinical study of intracranial atypical teratoid/rhabdoid tumors

目的探讨非典型畸胎样横纹肌样瘤（AT／RT）的临床特点、诊断及治疗方法，以提高对该疾病认识。方法回顾性分析本科经治且病理证实的1例患者的临床资料，并总结分析国内外此类疾病相关报道及研究。结果患者行手术治疗后术前剧烈头痛症状明显缓解，无手术相关并发症，出院后未行术后放化疗，随访患者5个月后死亡。结论AT／RT比较罕见，该类患者预后差，复发率及死亡率高，术后辅助放化疗能改善患者预后。

Objective To improve the cognition of the disease by exploring the clinical feature, diagnosis and therapeutic method of Atypical teratoid/rhabdoid tumors （AT/RT）. Method Clinical data of one patient diagnosed AT/RT by postoperative pathology was studied retrospectively, domestic and foreign related reports and studies of such disease were reviewed. Results The symptom of severe headache was obviously alleviated after surgical treatment, the patient followed- up abandoned postoperative radiation and chemotherapy and died after 5 months postoperatively. Conclusions AT/RT is a rare disease with poor prognosis, high recurrence and mortality. Postoperative adjuvant chemotherapy and radiation can improve the prognosis of patients.